restrictive lung disease Flashcards
- Nodule of WBCs formed as a reaction to infections, inflammation, irritants or foreign
bodies - Caseating vs non-caseating: necrotizing vs non-necrotizing
Granuloma
- the space between the air sacs and the small blood vessels that surround the air sacs
- It contains connective tissue
- oxygen from the air passes through your air sacs and lung interstitium and into the
blood
Interstitium
an air-containing space within a pulmonary infiltrate or mas
Cavitation
types of interstitial lung disease
exposure related: drug induced or occupational/environmental
autoimmune related
idiopathic (cause unknown)
- Histological and radiologic description of progressive lung scarring
- Patchy areas of diseased and healthy lung tissue—also temporal
- Honeycombing is almost 100% specific
Usual interstitial pneumonia
- Known causes vs. idiopathic–ascertaining the correct
diagnosis is important - The most common identifiable causes are exposure to occupational and environmental agents
- esp inorganic or organic dusts, drug-induced pulmonary toxicity, and radiation-induced lung injury
- can also complicate the course of most of the connective tissue diseases
- eg, polymyositis/dermatomyositis, rheumatoid arthritis, systemic lupus erythematosus, scleroderma,
mixed connective tissue disease
interstitial lung disease
work up steps of interstitial lung disease
Extensive History
* Age, gender, underlying comorbidities, drugs, smoking,
occupational history, hobbies, pets, family history, travel
- Duration of symptoms
- Physical Exam
Laboratories
* LFTs, RF, hypersensitivity panels, ANCA, Anti-glomerular
basement membrane antibody (Anti-GBM)
- Imaging
- Pulmonary function: Spirometry, lung volumes and DLCO
an umbrella term for a large group of disorders
that cause injury to lung tissue
* The scarring causes stiffness in the lungs which makes it difficult to breathe
* The fibrosis is irreversible
* Airway can be affected as well
Interstitial lung disease (ILD)
defined
by inhalation that fills the
lungs far less than would be
expected in a healthy person
restriction
how to make diagnosis of interstitial lung disease
- Insidious onset of progressive dyspnea and nonproductive chronic
cough - extrapulmonary findings may accompany specific diagnoses
- Tachypnea
- Small lung volumes
- Bibasilar dry rales
- Digital clubbing
- Right heart failure with advanced disease
- Chest Xray: low lung volumes and patchy distribution of ground
glass, reticular, nodular, reticulonodular, or cystic opacities - PFT:
- Reduced lung volumes
- Reduced pulmonary diffusing capacity–transfer of gas from air
in the lung to the red blood cells in lung blood vessels - Reduced 6-minute walk distance
- hypoxemia with exercise
what shape does restrictive lung disease show on flow volume loop
R
studies for interstitial lung disease that has infection or
indication of malignancy
Bronchoalveolar lavage (BAL)
how to confirm diagnosis of sarcoidosis
biopsy evidence of noncaseating granuloma from safest and most accessible
organ
- Varied, often nonspecific presentation
- Often asymptomatic, incidental finding
- Malaise, night sweats, fever, and dyspnea of insidious onset
- Cough, chest pain
- Physical findings are atypical of interstitial lung disease: crackles are
uncommon on chest examination - Skin involvement
- erythema nodosum, lupus pernio, iritis, peripheral neuropathy, arthritis, or
cardiomyopathy may also cause the patient to seek care - Other: parotid gland enlargement, hepatosplenomegaly, and
lymphadenopathy
Lupus Pernio
* Chronic raised indurated lesion
of the skin, often purplish in
color. It is seen on the nose,
ears, cheeks, lips, and forehead.
It is pathognomonic of this disease
Sarcoidosis: Symptoms and Signs
idiopathic pulmonary fibrosis treatment
- Managed by a pulmonologist
- Nintedanib and pirfenidone are FDA approved and reduce rate of decline of
lung function, but do not improve survival or QOL - Cost about $100,000 per year
- Oxygen
- Pulmonary rehab
- Monitor development of pulmonary hypertension
- Only definitive treatment is lung transplant
- 5 year survival rate estimated at 50%
systemic inflammatory disease of unknown etiology
* about 90% of patients have granulomatous inflammation of the lung
* Granulomatous nodules/collections of inflammatory cells can form in
multiple organs
* lung, lymph nodes, skin, eyes, peripheral nerves, liver, kidney, heart, and
other tissues can be involved
* Collection of mixed inflammatory cells
* Onset of disease is usually in the third or fourth decade
sarcoidosis
idiopathic pulmonary fibrosis CT scan shows
- predominantly basilar,
peripheral pattern of
dilated bronchiectasis - Reticulation
- Early honeycombing
- Diffuse Interstitial Pneumonia with no identifiable cause
- Diagnosis with 90% confidence in patients > 65 who have
- Idiopathic disease by history and who have inspiratory crackles
- Restrictive pathology on PFT
- Characteristic radiographic evidence of progressive fibrosis over
several years - and
- Diffuse, patchy fibrosis with pleural-based honeycombing on high
resolution CT scan - These patients do not need surgical lung biopsy
idiopathic pulmonary fibrosis
b/l hilar adenopathy alone (radiograph stage 1)
stage 1 sarcoidosis
parenchymal involvement alone, infiltrates only
stage 3 sarcoidosis
hilar adenopathy and parenchymal involvement
* Usually diffuse reticular infiltrates, but focal infiltrates,
bronchiole changes, nodules, pleural effusion and, rarely,
cavitation may be seen
stage 2 sarcoidosis
advanced fibrotic changes principally in the upper lobes
stage 4 sarcoidosis
- Chronic fibrotic lung diseases caused by the inhalation of inorganic dusts
- May be asymptomatic disorders with diffuse nodular opacities on CXR or may be
severe, symptomatic, life-shortening disorders - Treatment for all of these disorders is supportive
- Coal Miner’s: Simple and Complicated (Progressive massive fibrosis)
- Silicosis
- Asbestosis
Pneumoconioses
Sarcoidosis: Treatment
Indications for tx with oral
corticosteroids: disabling constitutional symptoms
- Prednisone, 20-40 mg/day for several weeks, with taper
- Long-term therapy is usually required over months to years
- Immunosuppressive medications, most commonly methotrexate,
azathioprine, or infliximab, are used in patients who are intolerant of
corticosteroids or who have corticosteroid-refractory disease
diagnosis of asbestosis
CXR showing linear streaking at the bases and opacities of varying shapes and sizes throughout the lungs
CXR – linear streaking at the lung bases, opacities of various shapes and sizes, honeycomb changes in advanced cases; pleural calcifications may also be present
CT scan of the chest is the best imaging study to detect fibrosis and pleural plaques
- Ingestion of inhaled coal dust by alveolar macrophages leads to the formation of coal macules that appear on CXR as diffuse small opacities
- most prominent in upper lungs
- Simple type – usually asymptomatic; pulmonary function is only slightly affected
- Cigarette smoking does not increase the prevalence of coal worker’s pneumoconiosis but may have an additive detrimental effect on ventilatory function
Complicated type – also known as progressive massive fibrosis
* conglomeration and contraction in the upper lung zones occur
Coal Worker’s Pneumoconiosis
Incidence of pulmonary TB is increased in patients with
silicosis
- All patients with silicosis should have a tuberculin skin test and
current chest XR
- Silicotic nodules caused by extensive or prolonged inhalation of silica particles
- A mineral found in rock and soil
- Exposure through construction and mining
- Small rounded opacities throughout the lung
Calcification of the periphery of hilar lymph nodes. scattered round
lung opacities
* “eggshell” calcifications: an unusual radiograph finding that strongly suggests silicosis
* Simple type– usually asymptomatic and does not affect PFTs
Complicated type
* large conglomerate densities appear in the upper lung
* dyspnea
* obstructive and restrictive pulmonary dysfunction
silicosis
- A nodular interstitial fibrosis occurring in workers exposed to asbestos fibers over
many years - usually 10-20
- shipyard and construction workers, pipe fitters, insulators
- First treatment is usually sought 15 years after initial exposure
- Signs/symptoms:
- Progressive dyspnea
- Inspiratory crackles
- Clubbing and cyanosis in some cases
asbestosis
- Also called extrinsic allergic alveolitis
- Nonatopic, nonasthmatic inflammatory pulmonary disease
- Caused by aerosolized exposure and sensitivity to organic
antigens: - agricultural dusts
- bioaerosols of microorganisms (fungal, bacterial, or protozoan)
- reactive chemical species
- Can be ACUTE or SUBACUTE or CHRONIC
- Usually a reversible course in the case of prompt diagnosis and removal
of the offending antigen - PFT: restriction and reduced DLCO
- Oral corticosteroids for severe or progressive disease
- soil/fertilizer
Hypersensitivity Pneumonitis
